Unexplained acidosis of malnutrition: a study by ion-exchange chromatography/mass spectrometry

Abstract

Keto-acidosis is usually associated with uncontrolled diabetes and typically poses few diagnostic problems when presenting as hyperglycaemia, metabolic acidosis and a high anion gap. An emaciated patient suffering from Duchenne Muscular Dystrophy and volume depletion presented with acidosis of unknown origin. Preliminary investigations appeared to rule out lactic acidosis, diabetic keto-acidosis and acidosis due to base loss. We have previously reported a technique utilizing liquid chromatography coupled to mass spectrometry (LC-MS) which can be used to characterize the underlying aetiology of acidosis and applied it to ultrafiltrate derived from a blood sample taken from this patient. The anion profile obtained on the chromatogram showed elevated levels of acetoacetate and hydroxybutyrate but no evidence of lactic acidosis, nor was the profile typical of that seen in 'unexplained' acidosis. We concluded that the patient was suffering from keto-acidosis associated with starvation and dehydration, the biochemical features being obscured by both the patient's chronic malnutrition and minimal muscle mass. A combination of enteral feeding and rehydration led to prompt resolution of the patient's metabolic acidosis.

Clinical review: Timing of renal replacement therapy

“…Renal failure results in increasing levels of plasma organic acids and other unmeasured anions through continued fixed acid production of around 50 to 100 meq H + /day [67-69]. RRT plays a major role in acid-base regulation in two ways, firstly by removing metabolic acids and secondly by the net addition of sodium bicarbonate.…”

Extreme Maternal Metabolic Acidosis Leading to Fetal Distress and Emergency Caesarean Section

“…Starvation ketoacidosis was described in two patients who were suffering from the Duchenne muscular dystrophy [9, 10]. However, one patient had a history of type 2 diabetes, and the other had a long-lasting history of dysphagia and malnutrition.…”

Short-term Effects of Acute Kidney Injury

“…Renal failure results in increasing levels of plasma organic acids and other unmeasured anions through continued fixed acid production of around 50 to 100 mEq H 1 /d. [28][29][30] Rapidly worsening high-aniongap acidoses in the context of AKI usually result from lactate generation caused by organ hypoperfusion, from ketoacids, or from poisoning, such as ethylene glycol or aspirin intoxication. Acidemia leads to protein catabolism, reduced hepatic blood flow, and hemodynamic instability caused by impairment of myocardial contractility, arteriolar dilatation, venoconstriction, and diminished response to catecholamines.…”